Journal of Scleroderma and Related Disorders最新文献

{"title":"Deficiency and altered phenotype of mucosal-associated invariant T cells in systemic sclerosis","authors":"Manon Lesturgie-Talarek, Virginie Gonzalez, L. Beaudoin, C. Frantz, Noémie Sénot, Zouriatou Gouda, Camille Rousseau, J. Avouac, A. Lehuen, Y. Allanore","doi":"10.1177/23971983231209807","DOIUrl":"https://doi.org/10.1177/23971983231209807","url":null,"abstract":"Systemic sclerosis is an autoimmune disease characterized by fibrosis of the skin and internal organs including the lung. Mucosal-associated invariant T cells are innate-like T lymphocytes able to produce various cytokines and cytotoxic mediators such as granzyme B. A large body of evidence supports a role of mucosal-associated invariant T cells in autoimmune disease but more recent reports suggest also a potential role in fibrotic conditions. Therefore, we herein addressed the question as whether mucosal-associated invariant T cells may have an altered profile in systemic sclerosis. Mucosal-associated invariant T cell frequency was analyzed by flow cytometry, using fresh peripheral blood from 74 consecutive systemic sclerosis patients who were compared to 44 healthy donors. In addition, in-depth mucosal-associated invariant T cell phenotype and function were analyzed in unselected 29 women with systemic sclerosis who were compared to 23 healthy women donors. Proportion of circulating mucosal-associated invariant T cells was significantly reduced by 68% in systemic sclerosis compared to healthy donors (0.78% in systemic sclerosis vs 2.5%, p < 0.0001). Within systemic sclerosis subsets, mucosal-associated invariant T cells were reduced in patients with interstitial lung disease (systemic sclerosis-interstitial lung disease) (0.56% vs 0.96% in patients without interstitial lung disease, p = 0.04). Moreover, in systemic sclerosis patients, mucosal-associated invariant T cells displayed an activated phenotype indicated by markedly increased CD69+ mucosal-associated invariant T cell frequency (20% mucosal-associated invariant T cell CD69+ compared to 9.4% in healthy donors, p = 0.0014). Interestingly, mucosal-associated invariant T cells from systemic sclerosis-interstitial lung disease patients had a more pronounced altered phenotype compared to systemic sclerosis without interstitial lung disease with a correlation between mucosal-associated invariant T cells expressing CCR6+ and mucosal-associated invariant T cell frequency (r = 0.8, p = 0.006). Circulating mucosal-associated invariant T cells were reduced and exhibited an activated phenotype in systemic sclerosis patients. This peripheral mucosal-associated invariant T cell deficiency may be related to enhanced apoptosis and/or homing in inflamed tissue, particularly in systemic sclerosis-interstitial lung disease patients.","PeriodicalId":506053,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":"92 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-11-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139246296","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Outcomes in systemic sclerosis patients hospitalized with COVID-19: Insight from the National Inpatient Sample","authors":"H. Cheema, A. Akhlaq, Biah Mustafa, A. Shahid, M. Ayyan, E. Edigin","doi":"10.1177/23971983231210340","DOIUrl":"https://doi.org/10.1177/23971983231210340","url":null,"abstract":"COVID-19, a respiratory infection caused by the novel coronavirus SARS-CoV-2, can cause varying degrees of illness ranging from mild respiratory illness to severe respiratory failure. Systemic sclerosis is a chronic autoimmune disease, with an increased prevalence of infections as compared to the general population. In this study, we compare the clinical outcomes and resource utilization for COVID-19 hospitalizations in patients with and without systemic sclerosis. We used the National Inpatient Sample database, 2020, to study the characteristics, morbidity, mortality, cost, and resource utilization among primary COVID-19 hospitalizations with and without systemic sclerosis. There were 1,050,040 patients aged ⩾ 18 years with a diagnosis of COVID-19. Of these, 775 (0.07%) patients had a secondary diagnosis of systemic sclerosis. Although there was no statistically significant difference regarding individual outcomes; in-hospital mortality, vasopressor use, cardiac arrest, acute kidney injury, and disposition to facility were numerically higher in hospitalizations with systemic sclerosis. The composite endpoint of major adverse events was higher in the systemic sclerosis cohort (adjusted odds ratio 1.52, 95% confidence interval: 1.06–2.17, p = 0.022). COVID-19 patients with systemic sclerosis had worse outcomes (i.e. higher composite endpoint of major adverse events) than those without systemic sclerosis. Further studies are needed to establish a better understanding of the relationship between COVID-19 and systemic sclerosis.","PeriodicalId":506053,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":"7 4","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-11-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139254053","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}